Acute Exacerbation of Interstitial Lung Disease: Definitions, Epidemiology, Prognosis and Management.

Abstract

Abstract Acute exacerbation of interstitial ling disease (AE-ILD) denotes a rapid deterioration of respiratory function often leading to ICU admission due to severe hypoxemia. Definitions encompass the diverse spectrum of interstitial lung diseases and specific criteria for AE-ILD, originally established for idiopathic pulmonary fibrosis (IPF) and later expanded to other interstitial lung diseases. The incidence varies among the various interstitial lung disease subtypes, with around 10% of interstitial lung disease patients experiencing acute exacerbations within the initial two years of diagnosis. Clinical risk factors encompass advanced interstitial lung disease, compromised pulmonary function, and coexisting conditions. Prognostic markers include baseline pulmonary parameters, blood markers, and a newly developed Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) staging system. However, due to the scarcity of randomized studies, optimal treatment strategies remain uncertain. Current guidelines propose supportive care and oxygen therapy, with cautious use of corticosteroids in AE-IPF. Antibiotics are employed to manage infections, with procalcitonin-guided therapy showing potential. Management involves exposure elimination, targeted antibiotics, and antacid and antifibrotic use. For severe cases, mechanical ventilation, extracorporeal membrane oxygenation (ECMO), and even transplantation are considered. To conclude, AE-ILD poses formidable challenges and high mortality rates. Given the lack of standardized approaches, the pursuit of randomized trials is vital to enhance the understanding and management of AE-ILD, optimizing patient outcomes within the ICU setting. Keywords: acute exacerbation, interstitial lung disease, idiopathic pulmonary fibrosis, intensive care unit, definition.